Abstract Number: PB1309
Meeting: ISTH 2020 Congress
Background: Moyamoya disease is a chronic progressive cerebrovascular condition characterized by bilateral stenosis/occlusion of the arteries around the circle of Willis, with prominent arterial collateral circulation. Etiology is unknown, but high incidence among Asian population and familial clustering in 10-15% suggest genetic predisposition.
Aims: Description of an occidental familial Moyamoya disease.
Methods: Collection of clinical data in SClínico® application.
Results: Male patient, 11 years-old, without Asian ancestry, with migraine antecedents, progressively presented, over the previous 24 hours, hypoesthesia and paresis of right upper limb, dysarthria, dysphagia to liquids and labial commissure deviation to the left side. Computed tomography and magnetic resonance imaging revealed recent ischemic injury in the left middle cerebral artery territory and alterations suggestive of Moyamoya disease. Therapeutic dose of enoxaparin was prescribed, with improvement of neurological deficits. Since discharge, patient has been accompanied by Neuropediatrics, Immunohemotherapy and Cardiology. Cerebral angiography confirmed diagnosis. Excepting patent foramen ovale (PFO) and interatrial communication (IAC), no other thrombotic risk factors were identified. Anticoagulation was maintained until percutaneous closure of PFO, posteriorly replaced by antiaggregation. Revascularization surgery was also performed.
The only sibling, male, 5 years-old, also with migraine history and no reported ischemic events, was evaluated. Likewise his brother, PFO, IAC and radiological alterations suggestive of Moyamoya disease were detected without other thrombotic risk factors. Antiaggregation was initiated.
Conclusions: Long-term antiaggregation is recommended for both asymptomatic and ischemic presentations. Anticoagulation as secondary prophylaxis remains controversial, due to increased bleeding risk, both in children (greater risk of falling) and adults (hemorrhage as predominant manifestation). This case emphasizes the importance of holistic management of thrombosis in Moyamoya disease (due to identification of PFO, anticoagulation was extended).
There are few studies that include non-Asian patients. Therefore, regional variants with prognostic/therapeutic implications cannot be excluded, highlighting the importance of reporting these cases and suggesting that multicenter trials may be needed.
To cite this abstract in AMA style:Vieira L, Pombal R, Lopes S, Ferreira D, Figueiredo M. Moyamoya Disease – A European Clinical Case Report [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/moyamoya-disease-a-european-clinical-case-report/. Accessed November 29, 2023.
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